肌痉挛性癫痫病相关EPM2A蛋白抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 肌痉挛性癫痫病相关EPM2A蛋白抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

Progressive myoclonic epilepsy type 2 (EPM2), also called Lafora disease, is an autosomal recessive disease characterized by grand mal seizures and/or myoclonus at about 15 years of age. Rapid and severe mental deterioration follows, often with psychotic features. Survival is less than 10 years after onset. Starch-like, endoplasmic reticulum-associated polyglucosans, called Lafora bodies, can be observed in brain, muscle, 肌痉挛性癫痫病相关EPM2A蛋白抗体liver and heart. One cause of Lafora disease is due to mutations in NHLRC1, the gene encoding Malin. Forty-nine different mutations in NHLRC1 have been shown to cause EPM2. Malin, also called NHL repeat-containing protein 1, is a single subunit E3 ubiquitin ligase, containing 6 NHL repeats and 1 RING-type zinc finger. Malin’s RING domain is responsible for its ability to mediate ubiquitination. Malin interacts with and polyubiquitinates 肌痉挛性癫痫病相关EPM2A蛋白抗体Laforin, a protein also implicated in EPM2. Malin localizes to the endoplasmic reticulum and, to a lesser extent, in the nucleus. Malin is expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas.

肌痉挛性癫痫病相关EPM2A蛋白抗体Also known as:

E3 ubiquitin-protein ligase NHLRC1; EPM2A; EPM2B; Malin; MGC119262; MGC119264; NHL repeat containing 1; NHL repeat containing protein 1; NHL repeat-containing protein 1; NHLC1_HUMAN; NHLRC 1; Nhlrc1.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Rabbit, .

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Immunogen: KLH conjugated synthetic peptide derived from human NHLRC1.

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肌痉挛性癫痫病相关EPM2A蛋白抗体Predicted Molecular Weight: 42kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-F=1:100-500  IF=1:50-200   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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